Evolving Approach in Hypoplastic Left Heart Syndrome With Restrictive and Intact Septum.

Background: Hypoplastic left heart syndrome (HLHS) with either intact atrial septum (IS) or highly restrictive interatrial communication (HRIC) is associated with poor survival. Immediate postpartum access to cardiac therapy and timely left atrial decompression (LAD) are paramount to a successful outcome. We describe herein our evolving approach to LAD and report interstage and longer-term results. Methods: We retrospectively identified neonates with HLHS IS/HRIC requiring LAD between 2005 and 2019. All babies had prenatal/postnatal echocardiography. Our LAD strategy evolved over time from attempt at transcatheter balloon atrial septostomy (BAS) to surgical septectomy with inflow occlusion, to hybrid trans-atrial stent implantation. Results: Twelve neonates required LAD at a median time of 14 (0.5-31) hours after birth. Five patients underwent BAS that proved successful in 2 cases. Of the 3 unsuccessful cases, 2 required extra-corporeal membrane oxygenation (ECMO) support and died subsequently; one underwent hybrid trans-atrial stent implantation. Of the remaining 7 patients, 3 underwent surgical septectomy with inflow-occlusion and 4 underwent hybrid trans-atrial stent implantations. Overall, 8 patients survived LAD and reached Norwood palliation. Three of the 8 required ECMO postoperatively. There was no hospital mortality after Norwood stage 1 palliation and interstage survival was 100%. Six patients successfully underwent Glenn shunt (superior cavopulmonary anastomosis) and 5 have completed total cavopulmonary connection. Conclusions: Our experience suggests that prompt postnatal LAD can be safely achieved with careful multidisciplinary planning and accurate antenatal diagnosis. In our hands, hybrid trans-atrial septal stent insertion appears to be a safe approach which combines the versatility of transcatheter techniques together with the effectiveness of surgical control.

What is the diagnostic value of the paediatric exercise tolerance test? Results from a UK centre.

Purpose The aim of this study was to determine whether the exercise tolerance test can provide diagnostic and prognostic information regarding children and young adults and help predict outcome. METHODS: A total of 87 patients, aged 7-29 years (median 13, mean 13.4) were selected retrospectively. They underwent exercise test at the Freeman Hospital from December, 2015 to May, 2016. There were two groups of patients - 46 had symptoms such as chest pain, palpitations, syncope, or dyspnoea on exertion and no cardiac diagnosis, and 40 patients had a cardiac diagnosis such as hypertrophic cardiomyopathy, transposition of the great arteries with post-arterial switch operation, aortic stenosis or regurgitation, tetralogy of Fallot, abnormal coronary arteries, Wolff-Parkinson-White syndrome, or supraventricular tachycardia. RESULTS: In the group of patients with symptoms and no cardiac diagnosis, exercise test was negative and there was no exercise-induced arrhythmia; 31 patients were discharged from follow-up. In the group of patients with a cardiac diagnosis, four patients had to be treated - one had ablation, one the Ross procedure, one aortic valve repair, and one aortic valve ballooning; in addition, seven patients had to be further investigated - one had signal average electrocardiogram, one stress cardiac MRI, two cardiac MRI, one lung function test, one reveal device, and one 24 hours electrocardiogram. In all, 43 patients were further followed-up from both groups. CONCLUSION: The exercise tolerance test is useful for clinical decision making in children and young adults with a cardiac diagnosis. In this study, the exercise tolerance test in patients with symptoms suggestive of cardiac disease but no cardiac diagnosis did not reveal any new diagnoses.